Cavernous malformation of the optic pathway mimicking optic glioma: a case report

Purpose

Optic pathway cavernous malformations (CMs) are extremely rare, accounting for less than 1 % of all intracranial CMs. We report a case of optic pathway CM mimicking optic glioma because the initial magnetic resonance (MR) images did not disclose hemorrhagic findings such as popcorn-like lesion or hemosiderin ring.

Methods

A 20-year-old woman presented with subacute left visual acuity loss and visual field defect and was referred to our hospital. Initial MR imaging findings were suggestive of optic glioma. Second MR imaging demonstrated hemorrhagic findings, but the hemorrhage was considered to be intratumoral hemorrhage from left optic pilocytic astrocytoma. She underwent radiochemotherapy and intravenous administration of corticosteroids but her symptoms deteriorated. Third and fourth MR imaging revealed enlargement of the hematoma. Therefore, the radiation therapy was interrupted at a delivered dose of 16.2 Gy, and craniotomy was performed to preserve residual right visual field. The lesion was totally removed and the histological diagnosis was CM. Her right visual field was preserved, but not improved.

Results

The present case and previous cases suggest that optic pathway CMs sometimes do not initially manifest with signs of hemorrhage, so CMs should be included in the differential diagnosis of optic pathway lesions.

Conclusions

Histological confirmation with preparation for total resection should be considered for rapidly progressive cases even if the neuroimaging findings are compatible with optic glioma.     

Thalamic arteriovenous malformation fed by the artery of Percheron originating from the contralateral posterior cerebral artery in a child

Purpose

Artery of Percheron (AOP) is a rare variant of thalamoperforating artery with a single common trunk feeding both thalami and with or without contribution to the rostral midbrain. We report the first case of thalamic arteriovenous malformation (AVM) fed by AOP with hemorrhagic onset.

Methods

A 12-month-old girl suddenly weakened and developed coma. Left thalamic hemorrhage was detected with the third and both lateral ventricles' hematoma. Thalamic AVM was discovered to be fed by an AOP originating from the contralateral posterior cerebral artery.

Results

Endovascular embolization of AVM was impossible due to a risk of bilateral thalamic infarction and anatomical inaccessibility. Thalamic AVM was removed by high superior parietal approach without new neurological symptoms.

Conclusion

The first case of thalamic AVM fed by AOP originating from the contralateral posterior cerebral artery is reported. Surgical removal of AVM would be at the heart of treatment in order to avoid bilateral thalamic infarction.     

Safety and efficacy using a detachable tip microcatheter in the embolization of pediatric arteriovenous malformations

Purpose

The treatment of deep-seeded pediatric brain arteriovenous malformations (AVMs) remains a challenging task. We describe our experience using a new detachable tip microcatheter in the embolization of brain arteriovenous malformations, pial arteriovenous fistulas, and vein of Galen malformations. We describe the safety and efficacy using a new detachable tip microcatheter in the treatment of pediatric deep brain arteriovenous malformations, pial malformations, and vein of Galen malformations.

Methods

During a period of 9 months from March 2013 through January 2014, 11 pediatric patients in 14 procedures with 27 total injections were selected for treatment with a detachable tip under Food and Drug Administration (FDA) compassionate use exemption and were admitted to our department for treatment of their brain AVM using a liquid embolic agent and a detachable tip microcatheter. The ages of the patients ranged from 3 months to 18 years old.

Results

Of the 27 total injections done, the tip detached in seven cases. For the 16 n-BCA injections, the tip detached six times (37.5 %), and for the 11 Onyx injections, the tip detached one time (9 %). There were no cases of premature microcatheter detachment during normal vessel navigation.

Conclusions

The introduction of these detachable tip microcatheters allows for a safe and relaxed injection that permits a true circumferential occlusion, and may further permit filling a larger amount of angioarchitecture without the risk of distal migration, or vessel damage during the usual rapid removal of non detachable micocatheters.     

Clinical features and endovascular treatment of intracranial arteriovenous malformations in pediatric patients

Purpose

The purpose of this study was to characterize clinical features and evaluate the clinical outcome of endovascular embolization treatment intracranial arteriovenous malformations in pediatric patients.

Methods

A cohort of children (age?≤?18 years) with arteriovenous malformations (AVMs) from 2000 to 2012 was included. Predictors studied included patient gender, age, and angioarchitectural features, including AVM location, nidus morphology and size, venous drainage, and associated aneurysms. Treatment method, complications and outcomes were recorded. The features of AVMs were evaluated before the treatment.

Results

One hundred twenty-seven children (77 males, mean age 13.2 years) were included; 90/127 (70.9 %) children were presented with hemorrhage. AVM size and deep venous drainage were independently associated with hemorrhage; 66/127 patients (52 %) treated with endovascular embolization. Complete obliteration at the end of all endovascular procedures was achieved in 14/66 patients (21.2 %), with an average of 78 % (range, 20–100 %) volume reduction. A mean of 2.9 (range, 1–9) feeding pedicles was embolized per patient. Overall, nine complications occurred in a total of 123 procedures (7.3 %). There was no procedure-related death in this study population. There was no significant difference between patients with and without complications in terms of AVM grade, demographic characteristics, or embolization features.

Conclusions

AVM size and deep venous drainage were independently associated with hemorrhage in pediatric patients. Endovascular procedure is feasible and safe for pediatric AVMs, and complete embolization can be achieved in small AVMs, while large AVMs can be adequately reduced in size for additional microsurgery or stereotactic radiosurgery.     

Dural sinus and internal jugular vein thrombosis complicating a blunt head injury in a pediatric patient

Introduction

Cerebral venous sinus thrombosis (CVST) following a blunt head trauma is a rare condition, described in the literature along with the lack of consensus regarding diagnosis and management.

Case summary

We present a case of a pediatric patient with a blunt head injury and epidural hematoma, who developed dural sinus and internal jugular vein thrombosis with fatal outcome.

Discussion

Most of reports show good outcome and recovery, but CVST might be related to poor recovery and even lead to death. The diagnosis and management of this condition are discussed based on a literature review.

Conclusion

It is important to keep a high degree of suspicion of CVST since early diagnosis may prevent potentially treatable catastrophic outcomes.     

Spontaneous spinal epidural hematoma mimicking Guillain-Barre Syndrome

Background

The initial symptoms of Guillain-Barre Syndrome (GBS) can be similar to a case of spontaneous spinal epidural hematoma (SSEH) located at the cervicothoracic junction. Therefore, SSEH may be misdiagnosed as GBS. Case Report: A previously healthy 6-year-old girl presented with a 2-day history of progressive pain in the lower extremities and an inability to walk. On initial evaluation, she was completely paraparetic in the lower extremities. Deep tendon reflexes were absent in the lower extremities, and Babinski reflexes were positive on both sides. She exhibited reduced response to light touch and pinprick with a sensory level below T10, and experienced difficulty during urination. However, the strength, sensation and flexion of upper extremities were normal. Because her presentation and examinations were consistent with GBS, we initiated intravenous immunoglobulin therapy. The next day, she also developed pain and muscle weakness of the right upper extremity. Three days after admission, respiratory depression progressed rapidly. Spinal MRI showed a mass extending from the level of C7-T3, with spinal cord compression. The patient underwent an emergency laminectomy with evacuation of hematoma, and was diagnosed with SSEH. Sixty days after admission, she was transferred to the rehabilitation hospital with severe neurologic sequelae of paralysis in both legs. Conclusion: SSEH might have severe consequences, including neurologic deficits and risk of death. This case report serves to raise the awareness of SSEH that mimics the initial presentation of GBS.     

Angiographic features help predict outcome after stereotactic radiosurgery for the treatment of pediatric arteriovenous malformations

Purpose

Arteriovenous malformations (AVMs) are a frequent cause of hemorrhagic stroke in children. Stereotactic radiosurgery (SRS) is an established treatment for these lesions, particularly those that are surgically inaccessible. Because only complete AVM obliteration is believed to protect against the future risk of hemorrhage, identifying lesion characteristics that predict response to therapy is an important objective. The goal of this study is to evaluate the influence of angiographic features of AVMs on the rate of obliteration following treatment with SRS.

Methods

This is a retrospective cohort study of pediatric patients (age ≤18 years) treated with Gamma Knife SRS for cerebral AVMs between 2000 and 2012. Detailed angiographic data at the time of initial angiographic evaluation were prospectively recorded by experienced neurointerventional radiologists. The primary outcome was the rate of obliteration on a 3-year follow-up angiogram.

Results

We identified 42 pediatric patients treated with SRS for cerebral AVMs. Twenty-seven patients completed 3-year angiographic follow-ups. Complete obliteration was seen in 30 %, partial response in 67 %, and no response in 4 %. Higher SRS dose was associated with complete obliteration. Larger AVM diameter, presence of multiple draining veins, and presence of multiple draining veins reaching a sinus were associated with partial response. In this small cohort, diffuse AVM borders, presence of aneurysm, and pre-SRS embolization were not associated with obliteration.

Conclusions

Our study identifies AVMs in the pediatric population with a nidus diameter of <2.5 cm and a solitary draining vein as the most likely to undergo complete obliteration after SRS treatment.     

Endovascular management of arteriovenous malformations and other intracranial arteriovenous shunts in neonates, infants, and children

Purpose

We discuss the management of cerebral arteriovenous shunts in neonates, infants, and children, with emphasis on our experience with pediatric cerebral arteriovenous malformations (AVMs). The management of vein of Galen malformations is discussed in a separate chapter.

Methods

An all-inclusive retrospective chart review of the endovascular surgery operative record database at the Hyman Newman Institute for Neurology and Neurosurgery at Roosevelt Hospital in NYC was conducted. All consecutive pediatric patients (newborn to 18 years of age) with intracranial arteriovenous shunts who presented from January 1, 2004 to June 16, 2009 were included.

Results

A total of 151 consecutive pediatric patients with intracranial arteriovenous shunts were evaluated from the period of January 1, 2004 to June 16, 2009. This included 56 patients with vein of Galen malformations, 48 cerebral AVMs, 11 patients with pial arteriovenous fistulae, six patients with dural arteriovenous malformations, and 30 patients with mixed intracranial vascular malformations. Forty-four patients underwent a total of 163 endovascular embolizations. The complications rate for endovascular embolizations was 6.7% (11 in 163), 5.5% with temporary complications and 1.2% with permanent complications. The mortality rate for the group of patients (excluding patients with vein of Galen malformations) that underwent endovascular embolizations was 0.0%.

Conclusions

Careful clinical observation and timely intervention are important in the management of pediatric patients with intracranial arteriovenous shunts. Trans-arterial endovascular embolization with liquid embolic agents is the treatment of choice for safe stabilization and/or improvement of symptoms in the group of pediatric patients with intracranial arteriovenous malformations.     

Conservative management of significant supratentorial epidural hematomas in pediatric patients

Background

Radiologically significant epidural hematomas are generally treated with craniotomy and evacuation. However, pediatric patients with such hematomas may have normal neurological examinations. We aim to report the presenting features, management, and outcomes of pediatric patients who underwent conservative management of such hematomas at our center and also show that serial neurological examinations are a safe and effective way of managing these patients.

Methods

A retrospective review of pediatric patients with extradural hematoma (EDH) thickness of at least 1 cm and who were conservatively managed was performed. All patients were followed up in neurosurgery outpatient clinics after discharge at 1-month, 3-month, 6-month, and 1-year intervals. Detailed neurological examination was performed at each visit, and a standardized questionnaire was also used to document the parent’s subjective opinion of the patient’s quality of life.

Results

A total of 17 patients satisfied the study criteria and were included. Conservative management was successful in 15 patients, while a craniotomy with evacuation of hematoma had to be performed in two patients. All patients had a GOS score of 5 on 1-year follow-up, had normal schooling, and reported complete satisfaction with the management protocol.

Conclusion

Conservative treatment is an optimal treatment option, and patients can be followed safely using a protocol of serial neurological examinations. A center must have resources to perform a craniotomy with evacuation of EDH in case of neurological worsening and be able to provide trained staff to carry out serial neurological examinations before treating these patients conservatively.     

Acute hemiparesis: A rare presentation of spontaneous spinal epidural hematoma mimicking acute stroke

Acute hemiparesis is an extremely rare presentation of spontaneous spinal epidural hematoma, which may be misdiagnosed as acute ischemic stroke and improperly treated with an intravenous thrombolytic agent. Here, we report a case of a 54-year-old woman who presented with acute neck pain and right-sided weakness. She was initially suspected of having ischemic stroke and therefore treated with an intravenous thrombolytic agent. However, she developed progressive tetraparesis, and subsequent magnetic resonance images confirmed cervical spontaneous spinal epidural hematoma.

Stroke is a leading cause of mortality and morbidity in Taiwan.1 Acute hemiparesis is one of the most indicative manifestations of acute stroke. Administration of recombinant tissue plasminogen activator (rtPA), a thrombolytic agent, within 3 to 4.5 hours of symptom onset is the standard treatment for acute ischemic stroke.2 However, various diseases such as slipped cervical discs, epilepsy, and spinal epidural hematoma may present along with acute hemiparesis, mimicking acute stroke, and thus be inappropriately treated with rtPA.3 Spontaneous spinal epidural hematoma (SSEH) is a rare condition of unknown etiology and requires urgent surgical intervention.3-5 The characteristic manifestation of SSEH is sudden-onset neck pain following by motor paralysis or tetraparesis.4 However, SSEH presenting with acute hemiparesis has been rarely reported in the literature. Therefore, we reported a case of SSEH with acute hemiparesis wrongly treated with rtPA in the emergency department (ED).     

Gamma Knife radiosurgery for arteriovenous malformations in pediatric patients

Objective

The authors present the results of Gamma Knife stereotactic radiosurgery performed in a series of children with arteriovenous malformations (AVMs).

Methods

Between June 2005 and January 2014, 75 patients 18 years old or younger received Gamma Knife radiosurgery for AVMs. Of these, 58 patients were eligible for further analysis. The median age of the population was 12 years; 41 % presented with hemorrhage, 34 % with neurological insult, and 24 % patients were diagnosed incidentally. The median AVM volume was 3.5 cm³. The median radiosurgery-based AVM score (RSBAVMS) was 0.86. The median follow-up period was 32 months.

Results

Single session Gamma Knife radiosurgery resulted in complete AVM obliteration in 40 (68.9 %) patients. There were 35 (60.3 %) excellent outcome (complete obliteration with no new deficits) in this series. During the follow-up period, nine (15.51 %) patients experienced new deficits and three (5.1 %) patients experienced intracranial hemorrhage. The annual rate of developing new deficits and hemorrhage was calculated as 5.45 and 1.8 %, respectively. Volume, gender, RSBAVMS, and nidus type factor were factors associated with excellent outcome.

Conclusions

Radiosurgery was successful in majority of patients with minimal morbidity. Gamma Knife radiosurgery for AVMs can be a safe and successful method in pediatric patients.     

Spinal epidural abscess from group A Streptococcus after varicella infection: a case report and review of the literature

Introduction

Spinal epidural abscess (SEA) is a very rare condition in pediatric patients. Varicella zoster infection could be a predisposing factor, and SEA should be suspected in patients with signs of secondary bacterial infection and even mild neurological signs.

Clinical case

We describe here a case of a 30-month-old girl with a history of remitting varicella infection, diagnosed for a lumbar epidural abscess and sacro-ileitis, secondary to group A Streptococcus (GAS).

Discussion

This is the third case of SEA from GAS reported in the literature in a pediatric population with varicella infection. We discuss here the clinical presentation and the diagnostic challenges for SEA in childhood through a review of the literature.     

Spontaneous spinal epidural haematoma mimicking meningitis in a 2-year-old child—a case report and literature review

Introduction

We report the case of a 2-year-old boy with suspected meningitis who presented with acute onset neck pain and stiffness associated with right-sided weakness and ataxia.

Management

Despite intravenous antibiotics and antiviral treatment, his condition deteriorated. Magnetic resonance imaging demonstrated spontaneous cervical epidural haematoma (C4–C7) extending down to thoracic (T7) level with associated compression of the spinal cord. He was treated successfully by neurosurgical decompression and made a complete recovery.

Discussion

Spinal epidural haematoma is a neurosurgical emergency characterised by extravasation of blood in the spinal epidural space. The clinical presentation particularly in young children can masquerade other conditions such as meningitis. In this article, we discuss our case and review the literature on spontaneous spinal epidural hematoma with an aim to improve awareness of this condition which if not recognised and treated early can lead to significant lifelong morbidity.     

Neuroimaging findings of retroclival hemorrhage in children: a diagnostic conundrum

Background

Retroclival hemorrhage in children may occur in three compartments, namely epidural, subdural, and subarachnoid, frequently secondary to trauma. Retroclival epidural hematoma may be associated with ligamentous injury, which may further result in instability at the craniocervical junction. Retroclival subdural hematoma may indicate a sentinel event for traumatic injury elsewhere within the brain or posterior fossa. Retroclival subarachnoid hemorrhage may have severe clinical consequences related to vasospasm.

Objective

Neuroimaging is essential in the recognition, localization, and characterization of retroclival hemorrhage into various compartments and for evaluating potential severe clinical consequences such as craniocervical junction instability, underlying traumatic brain injury, and ischemia secondary to vasospasm. The goal of this paper is to discuss the anatomy and biomechanics of the craniocervical junction as well as the neuroimaging findings associated with various compartments of retroclival hemorrhage in children.     

Multilevel thoracic hemangioma with spinal cord compression in a pediatric patient: case report and review of the literature

Purpose

Vertebral hemangiomas are common benign vascular tumors of the spine. It is very rare for these lesions to symptomatically compress neural elements. If spinal cord compression does occur, it usually involves only a single level. Multilevel vertebral hemangiomas causing symptomatic spinal cord compression have never been reported in the pediatric population to the best of our knowledge.

Methods

We report the case of a 15-year-old boy presenting with progressive paraparesis due to thoracic spinal cord compression from a multilevel thoracic hemangioma (T5–T10) with epidural extension.

Results

Because of his progressive neurological deficit, he was initially treated with urgent multilevel decompressive laminectomies from T4 to T11. This was to be followed by radiotherapy for residual tumor, but the patient was unfortunately lost to follow-up. He re-presented 3 years later with recurrent paraparesis and progressive disease. This was treated with urgent radiotherapy with good response. As of 6 months follow-up, he has made an excellent neurological recovery.

Conclusions

In this report, we present the first case of a child with multilevel vertebral hemangiomas causing symptomatic spinal cord compression and review the literature to detail the pathophysiology, management, and treatment of other cases of spinal cord compression by vertebral hemangiomas.     

Spinal cavernous extradural angiolipoma manifesting as a spontaneous spinal epidural hematoma in a child

Introduction

Angiolipomas are benign tumors usually manifested as subcutaneous nodules. Rarely they may form spinal extradural masses. These spinal cavernous angiolipomas have mostly been described in adults.

Diagnosis

To our knowledge, spinal cavernous angiolipomas have been reported in nine pediatric patients.

Clinical presentation

In this paper, we present a 1-year-old child presenting with a spontaneous spinal epidural hematoma from a spinal cavernous angiolipoma and highlight the importance of conducting a histopathological analysis of spontaneous hematomas in previously healthy children.

     

Spinal epidural venous angioma: a case report and review of the literature

Background

As a vascular malformation, venous angioma in the spinal cord is extremely rare. To our knowledge, there are only five case reports in the literature, and it has not been previously reported in the pediatric age group.

Case report

In this paper, we report on a 3-year-old patient who presented with progressive weakness in his left upper limb. Spinal magnetic resonance imaging (MRI) revealed an epidural cystic mass at the C6–T2 level. The lesion was diagnosed as venous angioma after total removal with laminectomy. Postoperatively, the patient remained symptom free, and no tumor recurrence was confirmed based on MRI at the time of the 18-month follow-up. The clinical, radiological, surgical, and pathological features of this abnormality are discussed, and all six reported cases were reviewed.

Conclusion

Venous angiomas should be included in the differential diagnosis of spinal cystic lesions in children. A definitive diagnosis is difficult based on MRI alone. This rare lesion is amenable to surgery, and gross total removal (GTR) is usually achievable due to a well-demarcated dissection plane. A good clinical outcome after GTR can be expected.     

Primary spinal epidural rhabdomyosarcoma: a case report and review of the literature

Background

Primary intraspinal sarcomas are very rare in children. Rhabdomyosarcoma (RMS) is a highly aggressive and rapidly growing sarcoma with skeletal origin that occasionally appears in the spinal epidural space.

Method

We report a 13-year-old girl who presented with back pain, progressive paraparesis, and urinary retention. She had muscular weakness in her lower extremities and absent deep tendon reflex. An epidural dumbbell-shape mass at T11–T12 level was observed on MRI. The patient underwent T12 hemilaminectomy, partial T11 hemilaminectomy, and right facetectomy. A large, firm, dark-red vascular epidural tumor was found compressing the cord. The tumor which extended to intracanal and foraminal parts was removed completely.

Discussion

Histopathological examination revealed undifferentiated small round and oval tumoral cells. Immunohistochemical staining was positive for vimentin, desmin, and myogenin. The final diagnosis was alveolar RMS. She received radiotherapy and chemotherapy by vincristine, actinomycin-D, doxorubicin, and ifosfamide. The patient was in good condition with no tumor recurrence or metastasis at the 1-year follow-up.     

Seizure and developmental outcomes after hemispherectomy in children and adolescents with intractable epilepsy

Purpose

The aim of this study is to describe a series of pediatric hemispherectomies, reviewing pathologic substrate, epilepsy characteristics and seizure outcome as well as developmental profiles, before and after surgery, in different domains.

Methods

Seventeen patients with full pre-surgical work-up, minimum follow-up of 12 months, and at least one post-surgical neuropsychological evaluation were selected. Three had Rasmussen encephalitis (RE), five hemispheric malformations of cortical development (MCD), and nine hemispheric vascular lesions.

Results

At latest follow-up, all patients with RE and 66.7 % of those with vascular lesions are in Engel’s class I; in the latter group, pre-surgical independent contralateral EEG discharges statistically correlated with a worse seizure outcome. Patients with MCD showed the worst seizure outcome. Pre-surgical language transfer to the right hemisphere was confirmed in a boy with left RE, operated on at 6 years of age. Patients with MCD and vascular lesions already showed severe global developmental delay before surgery, which persists afterwards. A linear correlation was found between earlier age at surgery and better outcome in personal–social, gross motor, and adaptive domains, in the vascular lesions group. The case with highest cognitive improvement had continuous spike and wave during sleep on pre-surgical EEG.

Conclusions

Pathologic substrate was the main factor related with seizure outcome. In children with MCD and vascular lesions, although developmental progression is apparent, significant post-surgical improvements are restricted by the severity of pre-surgical neuropsychological disturbances and a slow maturation. Early surgery assessment is recommended to enhance the possibilities for a better quality of life in terms of seizure control, as well as better autonomy and socialization.